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Α-SYNUCLEIN RT-QUIC IDENTIFIES LEWY BODY DISEASE WITH A HIGH ACCURACY IN BOTH CLINICAL PARKINSON’S DISEASE AND NEUROPATHOLOGICAL LEWY BODY DISORDERS
Abstract
Aims
To evaluate the performance of α-synuclein (α-syn) RT-QuIC in two independent cohorts.
Methods
α-syn-RT-QuIC was used to analyze lumbar CSF in a clinical cohort from the Swedish BioFINDER study and in post-mortem ventricular CSF in a neuropathological cohort from the Arizona Study of Aging and Neurodegenerative Disorders/Brain and Body Donation Program (AZSAND/BBDP). The neuropathological cohort included 101 individuals with different brain disorders, including Lewy Body disorders (LBD) and controls. The clinical cohort included 50 PD, 14 PDD, 15 MSA, 15 PSP, 47 controls and two individuals who were included as controls but who later converted to PD or DLB (LBD).
Results
BioFINDER cohort: 94.0% of PD and 100% of PDD patients were α-syn-RT-QuIC positive compared to 17.0% of the controls (non-LBD-converters), 33.3% of PSP and 33.3% of MSA patients. Both controls who later converted to LBD were α-syn-RT-QuIC positive. α-syn-RT-QuIC identified LBD (i.e. PD, PDD and converters) vs. controls with a sensitivity of 95.5%, a specificity of 83.0% and a diagnostic accuracy of 90.3%.
AZSAND/BBDP: α-syn-RT-QuIC could identify neuropathologically verified standard LBD, including PD, PD with Alzheimer’s disease (PDAD) and DLB (n=25) vs. no LB pathology (n=53) with a sensitivity of 100%, a specificity of 94.3% and a diagnostic accuracy of 96.2%. However, of the individuals with non-standard Lewy body disorders (n=23) including AD with Lewy body disease not meeting criteria for DLB or PD, and incidental LBD, only 56.5% were α-syn-RT-QuIC positive.
Conclusions
α-syn-RT-QuIC in the CSF is highly sensitive and specific for identifying subjects with clinical or clinicopathologically-defined Lewy body disorders.